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Proposed signaling events arising from CFTR dysfunction in PAECs in PAH. Cystic fibrosis transmembrane conductance regulator (CFTR); epithelial Na+ channels (ENaC); nitric oxide (NO); pulmonary arterial hypertension (PAH); pulmonary artery (PA); pulmonary artery endothelial cell (PAEC); reactive oxygen species (ROS).
